A CFTR corrector (lumacaftor) and a CFTR potentiator (ivacaftor) for treatment of patients with cystic fibrosis who have a phe508del CFTR mutation: a phase 2 randomised controlled trial (Q34662809)

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English	A CFTR corrector (lumacaftor) and a CFTR potentiator (ivacaftor) for treatment of patients with cystic fibrosis who have a phe508del CFTR mutation: a phase 2 randomised controlled trial	scientific article

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scholarly article

1 reference

Europe PubMed Central

PubMed publication ID

3 August 2017

A CFTR corrector (lumacaftor) and a CFTR potentiator (ivacaftor) for treatment of patients with cystic fibrosis who have a phe508del CFTR mutation: a phase 2 randomised controlled trial (English)

1 reference

Europe PubMed Central

PubMed publication ID

3 August 2017

phase II clinical trial

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cystic fibrosis

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1 reference

ChEBI release 2020-05-28

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ORCID Public Data File 2021

author name string

Michael P Boyle

1

1 reference

Europe PubMed Central

PubMed publication ID

3 August 2017

Scott C Bell

2

1 reference

Europe PubMed Central

PubMed publication ID

3 August 2017

Michael W Konstan

3

1 reference

Europe PubMed Central

PubMed publication ID

3 August 2017

Susanna A McColley

4

1 reference

Europe PubMed Central

PubMed publication ID

3 August 2017

Steven M Rowe

5

1 reference

Europe PubMed Central

PubMed publication ID

3 August 2017

Ernst Rietschel

6

1 reference

Europe PubMed Central

PubMed publication ID

3 August 2017

Xiaohong Huang

7

1 reference

Europe PubMed Central

PubMed publication ID

3 August 2017

David Waltz

8

1 reference

Europe PubMed Central

PubMed publication ID

3 August 2017

Naimish R Patel

9

1 reference

Europe PubMed Central

PubMed publication ID

3 August 2017

David Rodman

10

1 reference

Europe PubMed Central

PubMed publication ID

3 August 2017

VX09-809-102 study group

11

1 reference

Europe PubMed Central

PubMed publication ID

3 August 2017

publication date

24 June 2014

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Europe PubMed Central

PubMed publication ID

3 August 2017

The Lancet. Respiratory medicine

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Europe PubMed Central

PubMed publication ID

3 August 2017

2

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Europe PubMed Central

PubMed publication ID

3 August 2017

7

1 reference

Europe PubMed Central

PubMed publication ID

3 August 2017

527-538

1 reference

Europe PubMed Central

PubMed publication ID

3 August 2017

Cystic fibrosis

1 reference

https://api.crossref.org/works/10.1016%2FS2213-2600%2814%2970132-8

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Evidence for periciliary liquid layer depletion, not abnormal ion composition, in the pathogenesis of cystic fibrosis airways disease

1 reference

https://api.crossref.org/works/10.1016%2FS2213-2600%2814%2970132-8

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Targeting CFTR: how to treat cystic fibrosis by CFTR-repairing therapies.

1 reference

https://api.crossref.org/works/10.1016%2FS2213-2600%2814%2970132-8

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A CFTR potentiator in patients with cystic fibrosis and the G551D mutation

1 reference

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Rescue of CF airway epithelial cell function in vitro by a CFTR potentiator, VX-770

1 reference

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7 January 2021

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Ivacaftor in subjects with cystic fibrosis who are homozygous for the F508del-CFTR mutation

1 reference

https://api.crossref.org/works/10.1016%2FS2213-2600%2814%2970132-8

7 January 2021

based on heuristic

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Correction of the F508del-CFTR protein processing defect in vitro by the investigational drug VX-809.

1 reference

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Results of a phase IIa study of VX-809, an investigational CFTR corrector compound, in subjects with cystic fibrosis homozygous for the F508del-CFTR mutation.

1 reference

https://api.crossref.org/works/10.1016%2FS2213-2600%2814%2970132-8

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Cystic fibrosis transmembrane conductance regulator protein repair as a therapeutic strategy in cystic fibrosis

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Processing of CFTR: traversing the cellular maze--how much CFTR needs to go through to avoid cystic fibrosis?

1 reference

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Development and validation of The Cystic Fibrosis Questionnaire in the United States: a health-related quality-of-life measure for cystic fibrosis

1 reference

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A pilot clinical trial of oral sodium 4-phenylbutyrate (Buphenyl) in deltaF508-homozygous cystic fibrosis patients: partial restoration of nasal epithelial CFTR function

1 reference

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Evidence of CFTR function in cystic fibrosis after systemic administration of 4-phenylbutyrate

1 reference

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A phase I randomized, multicenter trial of CPX in adult subjects with mild cystic fibrosis

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Effect of aerosolized recombinant human DNase on exacerbations of respiratory symptoms and on pulmonary function in patients with cystic fibrosis. The Pulmozyme Study Group

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A controlled trial of long-term inhaled hypertonic saline in patients with cystic fibrosis

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Azithromycin in patients with cystic fibrosis chronically infected with Pseudomonas aeruginosa: a randomized controlled trial

1 reference

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Change in sweat chloride as a clinical end point in cystic fibrosis clinical trials: the ivacaftor experience

1 reference

https://api.crossref.org/works/10.1016%2FS2213-2600%2814%2970132-8

7 January 2021

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Mechanism-based corrector combination restores ΔF508-CFTR folding and function

1 reference

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Identifiers

10.1016/S2213-2600(14)70132-8

1 reference

Europe PubMed Central

PubMed publication ID

3 August 2017

PubMed publication ID

1 reference

Europe PubMed Central

PubMed publication ID

3 August 2017

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